Where on the Differential Is Eisenmenger Syndrome in a Patient Without Prior Cardiopulmonary Disease?

Abstract

Pulmonary hypertension (PH) can occur in patients with undiagnosed congenital heart disease, like atrial septal defects (ASDs), causing chronic left-to-right shunting. This may ultimately result in Eisenmenger physiology or syndrome (ES), a reversal of left-to-right shunting, resulting in a right-to-left shunt, thereby causing deoxygenated blood to enter systemic circulation as it bypasses the lungs. Development of PH due to an ASD is uncommon, and the occurrence of ES is <1% as most ASDs are corrected early in life. We present a 28-year-old female presenting with new-onset dyspnea found to have an undiagnosed ASD with ES.A 28-year-old female without past medical history presented to the emergency department after a chest x-ray performed by her primary care physician (PCP) showed dilation of the pulmonary artery concerning PH. The patient reported a three-month history of progressively worsening intermittent palpitations and dyspnea, now unable to walk more than one block without becoming dyspneic. Further imaging studies revealed a 1.4 centimeters (cm) secundum ASD, 4.4 cm dilatation of the PA, a mean pulmonary artery pressure (PAPm) of 132 millimeters (mm) mercury (Hg), and Eisenmenger physiology. She was placed on pulmonary vasodilators and iron supplementation to address an underlying iron-deficiency anemia. The patient is stable on her current regimen and is undergoing evaluation for possible heart-lung transplantation at an outside hospital.Dyspnea is one of the top 10 most common indications for emergency room visits annually. The differential diagnosis for dyspnea is vast, with ES, affecting only 0.8 in 1 million, far down on the list of possibilities, thus requiring high clinical suspicion to prompt further evaluation. Ultimately, the condition is preventable with early identification of underlying structural abnormalities for which definitive treatment options exist and are readily available, dramatically improving the prognosis if implemented before ES develops.

Síndrome de Eisenmenger secundario a comunicación interatrial tipo ostium secundum en mujer adulta

“…El cortocircuito de derecha a izquierda permite la entrada de sangre desoxigenada a la circulación sistémica, lo que provoca manifestaciones sistémicas como eritrocitosis, cianosis, intolerancia al ejercicio, palpitaciones, dedos en palillo de tambor, ingurgitación yugular, edema de miembros inferiores, livedo reticularis, entre otros 6 .…”

Síndrome de Eisenmenger en paciente adulto con disnea

“…del tabique interauricular, seno venoso y las aurículas comunes, La presión de la arteria pulmonar a nivel sistémico y el aumento del flujo de la arteria pulmonar provocan hipertrofia muscular de las arterias pulmonares pequeñas, lo que da como resultado una remodelación vascular pulmonar excesiva(van der Bruggen, Tedford, Handoko, van der Velden, & de Man, 2017).La terapia médica tradicional incluye bloqueadores de los canales de calcio, inhibidores de la fosfodiesterasa tipo 5 , antagonistas de la endotelina-1, modulación del óxido nítrico y agonistas de la prostaciclina. El objetivo de estos medicamentos es disminuir la vasoconstricción de la arteria pulmonar mediante la promoción de la relajación del músculo liso y los mecanismos vasodilatadores (Kellish, Hakim, Soal, Hassinger, & Gable, 2020).…”